All About Vulvar Cancer

What is vulvar cancer?

Vulvar cancer is an abnormal growth of malignant (cancerous) cells in the vulva. The vulva is defined as the external female genitalia, and includes the labia majora (outer lips of labia), labia minora (inner lips), clitoris, mons pubis, vestibule, or entryway, of the vagina, and the perineum (area between vulva and anus).

About 80% of vulvar cancers involve the labia, mainly the labia majora (~50%). About ten percent involve the clitoris, and another 10% involve the perineum, which is the area of sensitive skin located between the vagina and the anus. In about 5% of cases, the cancer is present at more than one site.

What are the different types of vulvar cancer?

The vulva is essentially epithelial skin; therefore the main tumor types that affect the vulva are skin-related cancers.

About 90% of vulvar cancers are squamous cell carcinomas, which typically develop at the edges of the labia majora/ minora or in the vagina. As with vaginal squamous cell carcinomas, vulvar squamous cell cancers are slow growing and usually develop from "precancerous", pre-invasive areas called vulvar intraepithelial neoplasia (VIN). There are two subtypes of squamous cell vulvar cancer. One is more common in younger women and is associated with the human papillomavirus (HPV); the other occurs in older women and is not associated with HPV infection, but is associated with chronic vulvar skin changes called vulvar dystrophy, including lichen sclerosus.

Melanoma is the second most common type of cancer found in the vulvar area and represents less than 5% of vulvar cancer cases. The most common skin-cancer in sun-exposed areas is basal cell carcinoma, and as expected, this type rarely occurs on the vulva.

A sarcoma of the soft tissue can develop in the vulva and these account for 1-2% of vulvar cancers. Adenocarcinomas of the vulva are also rare, but can develop from glands, most commonly the Bartholin's glands located at the vaginal opening.

How common is vulvar cancer?

Vulvar cancer is a relatively rare diagnosis, representing about 5% of all gynecologic cancers, and only about 1% of all female cancers in general. There are about 4850 new cases reported annually in the US and approximately 1030 deaths a year attributed to this disease.

What are the risk factors for vulvar cancer?

Vulvar cancer most commonly occurs in postmenopausal women. The peak age of diagnosis is between 70-79 years old. However, the rate of vulvar cancers diagnosed in younger women has been increasing in the last decade, as a result of vulvar cancers caused by Human Papilloma Virus (HPV) infection. These HPV-associated vulvar cancers are often seen in women under 45 years of age. Experts agree that these two groups of women have different types of squamous cell vulvar cancer, which behave differently and respond differently to treatment.

HPV Associated Vulvar Cancers

Between 40 and 60% of vulvar cancers are caused by HPV infection. HPV is a sexually transmitted disease that is incredibly common in the population. Most college-aged men and women have been exposed to HPV, though in most, the immune system inactivates or clears the virus from the body. There are over 100 different subtypes, or strains, of HPV and only certain subtypes are "oncogenic", or able to cause cancer (these include: HPV 16,18, 33, 39). Certain strains of HPV cause genital warts, though these strains are not oncogenic. Infection with HPV typically causes no symptoms, and may only be detected when a woman has an abnormal pap result or HPV testing that may be done along with the pap test. It is important to know that only a very small percentage of women who have a high-risk strain of HPV will develop a cancer caused by the virus; so simply having HPV does not mean that you will get cancer.

Women who have had multiple male sexual partners, began having sexual intercourse at an early age, or have had male sexual partners who are considered high risk (meaning that they have had many sexual partners and/or began having sexual intercourse at an early age) are at a higher risk for developing an HPV-related cancer.

Being a current smoker is also considered a risk factor. Smoking is linked to an inability for the body's immune system to clear an HPV infection; therefore smokers are more likely to develop chronic HPV infections that may lead to a cancer.

HPV-associated vulvar cancers may appear in more than one location and may occur in conjunction with cervical, vaginal or perianal cancers, as these are also caused by HPV infection.

Cases in Older Women

In post-menopausal women, vulvar cancer is often associated with long-term changes in the vulvar skin (vulvar dystrophy, squamous cell hyperplasia or lichen sclerosus). These may be a thickening or thinning of the vulvar skin or a white area that may be itchy or painful. Older women are less likely to attend preventive gynecologic healthcare visits and these conditions may go undiagnosed.

What are the symptoms of vulvar cancer?

The classic symptom is vulvar itching (pruritus), reported in almost 90% of the women with vulvar cancer. There can also be associated pain, pain with intercourse, bleeding, vaginal discharge, and/or painful urination (dysuria). Pre-cancerous lesions or early vulvar cancers may have mild or minimal symptoms. Preventive gynecologic exams can be helpful in detecting these early lesions.

Ultimately, many women will develop a visible vulvar mass: the squamous cell subtype can look like elevated white, pink or red bumps, while vulvar melanoma characteristically presents as a colored, ulcerated growth. There can be portions of the tumor that look sore and scaly, or cauliflower-like (similar to HPV-related warts).

How is vulvar cancer diagnosed?

First and foremost, a thorough gynecological examination should be performed using colposcopy and/or vulvoscopy, which uses a special magnifying instrument for better visualization. Any abnormal-appearing area(s) should be biopsied. Up to one-half of the time, the cancer may be "multi-focal"; meaning that the cancer is in two separate places. In addition, other HPV-related cancers can also be present (i.e. cervical, vaginal, perianal). Hence, a careful exam of all of the skin in the vaginal and groin area, as well as a gynecological exam should be performed. A Pap smear should be performed and additional smears taken from the vagina and vulva for testing.

CT scan or MRI of the abdomen/pelvis may be done to look for disease spread to lymph nodes and/or distant organs in advanced cases, but is not necessary in early stage disease. If spread to bladder or rectum is suspected, endoscopy (a scope to evaluate these areas; cystoscopy and proctoscopy, respectively) should be performed.

Once it is diagnosed, how is vulvar cancer staged?

Vulvar cancer can spread by direct extension, meaning that they can grow into adjacent areas such as the vagina and anus. Even in early disease, spread to lymph nodes can occur. However, spread to other organs is rare until late in the course of the disease.

The International Federation of Gynecology and Obstetrics (FIGO) uses a surgical staging system for vulvar cancer. This means that the stage of the cancer is not actually determined until after surgery is performed and the specimen is examined by the pathologist. This is because the presence of cancer in the inguinal lymph nodes (in the groin) is the most important factor in determining prognosis and these nodes cannot accurately be evaluated without surgery.

How is vulvar cancer treated?

Surgery, radiation therapy and chemotherapy are all treatment options, and are typically used in various combinations. Surgery techniques used today are less radical than previously used and therapy choices are individualized, based on each woman's case. As with many cancers, the optimal treatment depends on the tumor size, location, lymph node involvement and patient factors such as age and other medical conditions. Every woman should have a thorough discussion with their gynecologic oncologist about the options for treatment and the benefits and risks of these options.

Possible treatment options by stage are as follows:

Stage I

Radical local excision (a deep excision of the tumor) with or without removal of all nearby groin/ upper thigh lymph nodes or sentinel lymph node biopsy.

Modified radical vulvectomy and removal of groin lymph nodes on both sides of the body or sentinal lymph node biopsy. Postoperative radiation therapy to the pelvis with or without chemotherapy if lymph nodes are positive for cancer.

Radiation therapy alone (in selected patients).

Stage III

Radical vulvectomy and removal of groin/ upper thigh lymph nodes on both sides of the body, plus postoperative radiation therapy to the pelvis and groin if lymph nodes are positive for cancer or if the primary vulvar tumor is very large.

Neoadjuvant chemoradiation (chemotherapy and radiation given prior to surgery) is often used in advanced cancers to shrink the tumor in order to minimize the surgery needed or eliminate the need for surgery altogether.

Radiation therapy (in selected patients) with or without chemotherapy.

Stage IV

Neoadjuvant chemoradiation (chemotherapy and radiation given prior to surgery) is often used in advanced cancers to shrink the tumor in order to minimize the surgery needed or eliminate the need for surgery altogether.

Pelvic exenteration is used rarely due to the extent of the surgery and the effect on quality of life. Exeneration entails radical vulvectomy and removal of the lower colon, rectum, or bladder (depending on where the cancer has spread), as well as the uterus, cervix, and vagina.

Radiation therapy with chemotherapy, with or without surgery.

Follow Up Care

After treatment, following up with your physician is essential. It is recommended that after treatment, a gynecologic examination should be performed at least twice a year with careful inspection of the skin and inguinal lymph nodes (by palpation- or feeling them). If any abnormalities are seen, a colposcopy and biopsy should be performed. Up to 10% of recurrences can occur after 5 years, underscoring the importance of long follow up.

After treatment, talk with your oncology team about receiving a survivorship care plan, which can help you manage the transition to survivorship and learn about long-term concerns and life after cancer. You can create your own survivorship care plan on OncoLink.

What is the prognosis?

Prognosis depends on a number of factors, including the presence or absence of cancer in lymph nodes, the number of involved lymph nodes, if the cells spread outside the positive lymph node(s), and tumor size. In addition the category of squamous cell cancer – is this an HPV-associated cancer or a cancer diagnosed in an older woman that is HPV negative? What treatment(s) can the patient tolerate? Because the prognosis relies on many factors, all women should have a conversation with their gynecologic oncologist about their particular case.

Recurrence of Vulvar Cancer

Local recurrence (near where the cancer was originally found) is the most common place where cancers can recur. These can often be treated with surgery. If the disease has spread to other organs (metastatic or distant disease), it may be treated with chemotherapy. The most frequently used chemotherapy regimens are called "platinum-based", meaning they consist of cisplatin, given alone or combined with another agent, such as 5-FU, paclitaxel, vinorelbine or mitomycin C. There is no "standard" treatment because there are so few cases, clinical trials to identify the best regimen are limited. The response rate to these chemotherapies is low and the length of response tends to be brief. There is ongoing research examining the use of new biological agents, such as gefitinib and erlotinib, which appear to have good early results.

Side Effects of Vulvar Cancer & Cancer Treatment

Many of the side effects from surgery and radiation occur due to the close proximity of the bladder and rectum to the vulva. Due to this close proximity, these organs can be damaged during surgery or with radiation. Side effects from the radiation can include irritation of the bowel and bladder resulting in diarrhea and increased frequency or urgency of bowel movements or urination. This typically resolves within a few weeks of finishing treatment, though it can become a long-term concern for some women.

Radiation can cause scar tissue to form in the vagina and the tissue can become dry and less elastic. There may be some shrinking of the vagina and vaginal opening. Scarring of the vaginal tissue can result in "adhesions", or areas where scar tissue forms, sealing the sides of the vaginal together. This can make it difficult for the doctor to perform vaginal exams and makes sexual intercourse difficult and uncomfortable. Your oncology team will teach you to use vaginal dilators to reduce the severity of this side effect. Rarely, a connection between the bladder or rectum and the vagina can form (also known as a fistula), which allows passage of stool or urine into the vagina.

Damage to the drainage (lymphatic) system in the area, by radiation or surgery to remove lymph nodes, can lead to a chronic swelling called lymphedema, which can occur at any time after treatment. Studies have found this condition to occur in anywhere between 14-48% of women who undergo lymph node dissection for vulvar cancer. Notify your healthcare provider if you develop any swelling in the legs or pelvis. A survivor with lymphedema who develops pain or redness in the leg(s), especially with fever, should be evaluated right away, as these signs may indicate infection. Some women may be good candidates for sentinel lymph node biopsy (SLNB), which appears to greatly reduce the risk of developing lymphedema. In SLNB, only the lymph nodes that the tumor area drains to are removed, limiting the damage done to the lymph system. SLNB is performed by gynecologic oncologists that have been trained in this procedure.

More than half of the women who undergo vulvectomy report sexual dysfunction and psychological issues. The extreme changes in a woman's anatomy can result in painful intercourse, body image concerns, decreased desire, inability to orgasm, and difficulty with urination. Adequately preparing a woman for the expected changes, providing tools for coping with these changes, and the discussing the emotions surrounding the cancer and its treatment may help some women. Women should not hesitate to talk openly with their healthcare practitioners about their feelings and fears and consider professional counseling to help in healing emotionally, as well as physically.

References & Further Resources

Eyes On The Prize: information and emotional support for those affected by gynecologic cancers. Has a helpful discussion board where you can "chat" with other women.

Pecorelli S. Revised FIGO staging for carcinoma of the vulva, cervix, and endometrium. International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics. May 2009;105(2):103-104.